72939Prince Aly Khan Hospital, Mumbai, Maharashtra, India.
Int J Surg Pathol. 2022 Apr;30(2):190-194. doi: 10.1177/10668969211027409. Epub 2021 Jun 28.
A 68-year-old male presents with generalized lymphadenopathy and fever of short duration. Axillary lymph node excision was performed and was sent for histopathological evaluation. Microscopic evaluation of the submitted lymph node revealed diffuse proliferation of intermediate-sized atypical lymphoid cells with round nuclei, irregular membranes, finely dispersed chromatin, and inconspicuous nucleoli. Mitotic figures were frequently seen. Immunohistochemical evaluation revealed diffuse expression of CD20, CD5, CD10, B-cell lymphoma 2 (Bcl2), and B-cell lymphoma 6 (Bcl6). Atypical lymphoid cells were negative for cyclin D1; however, showed diffuse and strong nuclear expression of SOX11. MIB1 proliferation index was high (Ki67: 90%-95%). Based on morphological features and immunohistochemical findings a diagnosis of "cyclin D1 negative aggressive blastoid variant of mantle cell lymphoma (MCL)" was offered. The classic morphology of MCL is seen in 90% of cases, while the remaining (∼10%) are considered as variants. A blastoid variant is an aggressive subtype that can lack expression of CD5 as well as cyclin D1, but instead expresses CD10, Bcl6, and CD23. SOX11 expression is seen in 90% cases of MCL and in almost 100% cases of cyclin D1 negative MCL. The current case highlights the unusual morphologic and aggressive variant of MCL and a significant role of SOX11 in its diagnosis.
一位 68 岁男性因全身淋巴结病和短期发热就诊。进行了腋窝淋巴结切除术，并将其送检进行组织病理学评估。送检淋巴结的显微镜评估显示中等大小的异型淋巴样细胞弥漫性增生，细胞核圆形，细胞膜不规则，染色质细分散，核仁不明显。有丝分裂象频繁可见。免疫组织化学评估显示弥漫性表达 CD20、CD5、CD10、B 细胞淋巴瘤 2（Bcl2）和 B 细胞淋巴瘤 6（Bcl6）。异型淋巴细胞 cyclin D1 阴性，但弥漫性和强核表达 SOX11。MIB1 增殖指数高（Ki67：90%-95%）。根据形态学特征和免疫组织化学结果，诊断为“cyclin D1 阴性侵袭性母细胞样变异型套细胞淋巴瘤（MCL）”。MCL 的典型形态在 90%的病例中可见，而其余（约 10%）被认为是变异型。母细胞样变体是一种侵袭性亚型，可能缺乏 CD5 和 cyclin D1 的表达，但表达 CD10、Bcl6 和 CD23。SOX11 在 90%的 MCL 病例中表达，在几乎 100%的 cyclin D1 阴性 MCL 病例中表达。本病例突出了 MCL 的罕见形态和侵袭性变异型，以及 SOX11 在其诊断中的重要作用。
